Florida Atlantic University Charles E. Schmidt College of Medicine, Boca Raton, USA.
Department of Neurosurgery, University of Miami Miller School of Medicine, Miami, USA.
World Neurosurg. 2020 Jun;138:498-503. doi: 10.1016/j.wneu.2019.12.084. Epub 2019 Dec 24.
Gangliogliomas are rare, well-differentiated, low-grade neoplasms that most often occur unifocally in children and most commonly affect the temporal lobe. Gangliogliomas that occur in patients age >40 years tend to have worse prognoses. These tumors generally stain positively for neural and glial cell markers, as well as CD34. Here we report an unprecedented case of multifocal intracranial ganglioglioma in an adult age >40 who had a favorable course, and review the current literature on multifocal intracranial gangliogliomas.
A 60-year-old female presented to her ophthalmologist with blurry vision in the right eye and an unremarkable neurologic exam. She was referred for brain imaging, which showed multiple lesions in both cerebral hemispheres. Biopsy of the right occipital lesion was elected, as it enhanced the most on magnetic resonance imaging.
Multifocal intracranial gangliogliomas are exceedingly rare tumors, especially in adults. These tumors present unique management barriers because as they are multifocal at the time of diagnosis, making resection more technically challenging. In our review, the average age at diagnosis was 19.2 years, and 80% of the cases had at least 1 lesion in the temporal lobe. Two studies opted for resection of intracranial tumors, whereas the remaining studies performed biopsy with conservative management and serial imaging. Biopsy was performed in all cases. We present the first case of an intracranial multifocal ganglioglioma in a patient age >40 years with lesions in the occipital lobe, corpus callosum, and frontal lobe at presentation.
神经节细胞瘤是一种罕见的、分化良好的低度恶性肿瘤,多发生于儿童,且最常累及颞叶。年龄>40 岁的患者发生的神经节细胞瘤预后较差。这些肿瘤通常对神经和神经胶质细胞标志物以及 CD34 呈阳性染色。在此,我们报告了首例>40 岁成人多灶性颅内神经节细胞瘤病例,该患者病情良好,并对目前多灶性颅内神经节细胞瘤的文献进行了回顾。
一名 60 岁女性因右眼视力模糊和神经检查无明显异常而就诊于眼科医生。她因脑部影像学检查显示双侧大脑半球有多个病变而被转诊。由于右侧枕叶病变在磁共振成像上增强最明显,因此选择对其进行活检。
多灶性颅内神经节细胞瘤是非常罕见的肿瘤,尤其是在成人中。这些肿瘤具有独特的管理障碍,因为它们在诊断时即为多灶性,使得手术切除更具挑战性。在我们的回顾中,平均诊断年龄为 19.2 岁,80%的病例至少在颞叶有 1 个病灶。有 2 项研究选择了颅内肿瘤切除术,而其余研究则进行了活检和保守治疗以及连续影像学检查。所有病例均进行了活检。我们报告了首例>40 岁成人多灶性颅内神经节细胞瘤病例,患者在就诊时即存在枕叶、胼胝体和额叶的病变。